Magnetic resonance imaging based kidney volume assessment for risk stratification in pediatric autosomal dominant polycystic kidney disease

IntroductionIn the pediatric context, most children with autosomal dominant polycystic kidney disease (ADPKD) maintain a normal glomerular filtration rate (GFR) despite underlying structural kidney damage, highlighting the critical need for early intervention and predictive markers. Due to the inverse relationship between kidney volume and kidney function, risk assessments have been presented on the basis of kidney volume. The aim of this study was to use magnetic resonance imaging (MRI)-based kidney volume assessment for risk stratification in pediatric ADPKD and to investigate clinical and genetic differences among risk groups.MethodsThis multicenter, cross-sectional, and case-control study included 75 genetically confirmed pediatric ADPKD patients (5–18 years) and 27 controls. Kidney function was assessed by eGFR calculated from serum creatinine and cystatin C using the CKiD-U25 equation. Blood pressure was assessed by both office and 24-hour ambulatory measurements. Kidney volume was calculated from MRI using the stereological method. Total kidney volume was adjusted for the height (htTKV). Patients were stratified from A to E classes according to the Leuven Imaging Classification (LIC) using MRI-derived htTKV.ResultsMedian (Q1-Q3) age of the patients was 6.0 (2.0–10.0) years, 56% were male. There were no differences in sex, age, height-SDS, or GFR between the patient and control groups. Of the patients, 89% had PKD1 and 11% had PKD2 mutations. Non-missense mutations were 73% in PKD1 and 75% in PKD2. Twenty patients (27%) had hypertension based on ABPM. Median htTKV of the patients was significantly higher than controls (141 vs. 117 ml/m, p = 0.0003). LIC stratification revealed Classes A (38.7%), B (28%), C (24%), and D + E (9.3%). All children in class D + E and 94% in class C had PKD1 variants. Class D + E patients had significantly higher blood pressure values and hypertension compared to other classes (p > 0.05 for all).DiscussionThis study distinguishes itself by using MRI-based measurements of kidney volume to stratify pediatric ADPKD patients into specific risk groups. It is important to note that PKD1 mutation and elevated blood pressure were higher in the high-risk groups stratified by age and kidney volume. Our results need to be confirmed in further studies

Global Variation of Nutritional Status in Children Undergoing Chronic Peritoneal Dialysis : A Longitudinal Study of the International Pediatric Peritoneal Dialysis Network

While children approaching end-stage kidney disease (ESKD) are considered at risk of uremic anorexia and underweight they are also exposed to the global obesity epidemic. We sought to investigate the variation of nutritional status in children undergoing chronic peritoneal dialysis (CPD) around the globe. The distribution and course of body mass index (BMI) standard deviation score over time was examined prospectively in 1001 children and adolescents from 35 countries starting CPD who were followed in the International Pediatric PD Network (IPPN) Registry. The overall prevalence of underweight, and overweight/obesity at start of CPD was 8.9% and 19.7%, respectively. Underweight was most prevalent in South and Southeast Asia (20%), Central Europe (16.7%) and Turkey (15.2%), whereas overweight and obesity were most common in the Middle East (40%) and the US (33%). BMI SDS at PD initiation was associated positively with current eGFR and gastrostomy feeding prior to PD start. Over the course of PD BMI SDS tended to increase on CPD in underweight and normal weight children, whereas it decreased in initially overweight patients. In infancy, mortality risk was amplified by obesity, whereas in older children mortality was markedly increased in association with underweight. Both underweight and overweight are prevalent in pediatric ESKD, with the prevalence varying across the globe. Late dialysis start is associated with underweight, while enteral feeding can lead to obesity. Nutritional abnormalities tend to attenuate with time on dialysis. Mortality risk appears increased with obesity in infants and with underweight in older children.Peer reviewe

The crush syndrome

Crush syndrome is a systemic disorder resulting from trauma-associated rhabdomyolysis that causes several medical and surgical complications. Pathogenesis of crush syndrome-related acute renal failure (ARF) can be studied under two headings: 1) Rhabdomyolysis, and 2) ARF on the basis of rhabdomyolysis. Compression of the muscles (baromyopathy) induces rhabdomyolysis. The term rhabdomyolysis points out to the disintegration of striated muscles, which results in the release of muscular cell contents into the circulation. On the other hand sodium, chloride, water and calcium diffuse into the muscle cell, and cellular swelling, "compartment syndrome", occurs. Various factors contribute to the development of ARF on the basis of rhabdomyolysis. Among these the most important one is impairment of renal perfusion as a result of hypovolemia due to compartment syndrome. The crush syndrome and ARF do not necessarily develop in all cases who suffer from muscle trauma. This syndrome is observed relatively rarely in children. The most useful indicator of rhabdomyolysis is an increase in serum creatine phosphokinase level. On the other hand the most critical laboratory abnormality is hyperkalemia. Considering the medical interventions, prophylaxis of ARF is vital. Fluid replacement is the most important measure for this goal and intermittent hemodialysis is the most effective modality for the treatment of the crush patients with ARF. (Turk Arch Ped 2009; 44: 43-7

Renal Crisis in Children during Armed Conflict

Armed conflicts continue to occur in some regions of the globe, mostly in developing countries. These man-made disasters affect all segments of the population; however, some groups are more vulnerable and suffer more seriously from the unfavorable consequences of such conflicts. Among these, the pediatric population deserves special attention because they cannot protect themselves, and hence carry a higher threat of injuries and probability of death during conflicts. In addition, children who do survive the disaster are more prone to exploitation. Pediatric victims, including those who sustain acute kidney injury or those suffering from chronic kidney disease before armed conflicts, face higher risks of morbidity and mortality as a result of treatment problems, specifically limited dialysis options. Displaced children, forced to flee their homes as a result of armed conflicts, are also at risk for various health problems because they may not find ideal circumstances for disease treatment. Making preparations in anticipation of armed conflicts, such as disaster-relief scenarios and action plans, may be useful to decrease the death toll in these children, who are dependent on their caregivers for survival. Adopting principles of disaster nephrology may contribute to improved survival chances of pediatric kidney patients in chaotic circumstances. (C) 2020 Elsevier Inc. All rights reserved

Disasters, children and the kidneys

Following disasters, children are physically, psychologically and socially more vulnerable than adults; consequently, their morbidity and mortality are higher. The risks are especially high for orphans and unaccompanied children who are separated from their families, making them frequently victims of human trafficking, slavery, drug addiction, crime or sexual exploitation. Education of children and families about disaster-related risks and providing special protection in disaster preparedness plans may mitigate these threats. Kidney disease patients, both paediatric and adult, are extra vulnerable during disasters, because their treatment is dependent on technology and functioning infrastructure. Acute kidney injury, chronic kidney disease patients not on dialysis and dialysis and transplant patients are faced with extensive problems. Overall, similar treatment principles apply both for adults and paediatric kidney patients, but management of children is more problematic, because of substantial medical and logistic difficulties. To minimize drawbacks, it is vital to be prepared for renal disasters. Preparedness plans should address not only medical professionals, but also patients and their families. If problems cannot be coped with locally, calling for national and/or international help is mandatory. This paper describes the spectrum of disaster-related problems in children and the specific features in treating acute and chronic kidney disease in disasters

Abdominal manifestations of polyarteritis nodosa demonstrated with CT

We report a rare case of polyarteritis nodosa (PAN) presenting in childhood. The child had multiple visceral aneurysms and later developed ascending colitis and jejunitis. The diagnosis was established with multidetector CT and CT angiography